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Skin of color (SOC) patients are projected to comprise the majority of the population by 2044, yet knowledge gaps in the clinical presentation and treatment of both common and uncommon dermatologic conditions in skin of color persist. Improved awareness of disparities that disproportionately impact SOC patients is necessary to address health inequity in the field of dermatology. The first part of this CME discussed structural, genetic, and immunophenotypic differences in SOC in common inflammatory disorders as well as cutaneous malignancies. The second part of this CME highlights clinical differences in the phenotypic presentation of the inflammatory disorders of atopic dermatitis, psoriasis, and hidradenitis suppurativa as well as the cutaneous malignancies of melanoma, basal cell carcinoma, and cutaneous T-cell lymphoma. Health disparities associated with each of these conditions are also discussed. [ABSTRACT FROM AUTHOR]

Skin of color (SOC) populations include those who identify as Black/African, Hispanic/Latinx, Asian/Pacific Islander, American Indian/Native Alaskan, Indigenous Australian, Middle Eastern, biracial/multiracial, or non-White; this list is far from exhaustive and may vary between and within cultures. Recent genetic and immunological studies have suggested that cutaneous inflammatory disorders (atopic dermatitis, psoriasis, and hidradenitis suppurativa) and malignancies (melanoma, basal cell carcinoma, and cutaneous T-cell lymphoma) may have variations in their immunophenotype among SOC. Additionally, there is growing recognition of the substantial role social determinants of health play in driving health inequalities in SOC communities. It is critically important to understand that social determinants of health often play a larger role than biologic or genetic factors attributed to "race" in health care outcomes. Herein, we describe the structural, genetic, and immunological variations and the potential implications of these variations in populations with SOC. This article underscores the importance of increasing the number of large, robust genetic studies of cutaneous disorders in SOC to create more targeted, effective therapies for this often underserved and understudied population. Part II of this CME will highlight the clinical differences in the phenotypic presentation of and the health disparities associated with the aforementioned cutaneous disorders in SOC. [ABSTRACT FROM AUTHOR]

Background: Patients with cutaneous T-cell lymphoma (CTCL) are at a higher risk of developing second malignancies. However, rates of incidence vary significantly across studies.Methods: A systematic review and meta-analysis of articles published between 1950 and 2019 was performed to evaluate the risk of second malignancies in patients with CTCL.Results: We identified 10 eligible studies, including 12 patient cohorts, with 5.9% to 16.8% of patients developing second malignancies. All studies showed a male predominance for patients developing second malignancies. The mean age across the studies ranged from 44.6 to 68.0 years. The time between the diagnosis of CTCL and second malignancy ranged from 2.1 to 5.4 years (mean, 3.29 y; 95% confidence interval [CI], 2.69-5.15). Meta-analysis showed a standardized incidence ratio of 2.18 (95% CI, 1.43-2.93) for all malignancies. The standardized incidence ratios were 15.25 (95% CI, 7.70-22.79) for Hodgkin lymphoma, 4.96 (95% CI, 3.58-6.33) for non-Hodgkin lymphoma, 1.69 (95% CI, 1.18-2.21) for lung cancer, 1.72 (95% CI, 1.18-2.21) for bladder cancer, and 3.09 (95% CI, 1.77-6.43) for melanoma.Conclusions: We find that patients with CTCL are at increased risk of second malignancies, especially Hodgkin and non-Hodgkin lymphoma, lung cancer, bladder cancer, and melanoma. These findings provide evidence of a population at increased risk of malignancy. Early detection may decrease the morbidity burden of second malignancies, thus providing a strong rationale for prospective screening studies. [ABSTRACT FROM AUTHOR]

Palliative care has been shown to improve quality of life, symptoms, and caregiver burden for a range of life-limiting diseases. Palliative care use among patients with severe dermatologic disease remains relatively unexplored, but the limited available data suggest significant unmet care needs and low rates of palliative care use. This review summarizes current palliative care patterns in dermatology, identifying areas for improvement and future investigation. [ABSTRACT FROM AUTHOR]

Cutaneous T cell lymphomas (CTCLs) are malignancies of skin-trafficking T cells. Patients with advanced CTCL manifest immune dysfunction that predisposes to infection and suppresses the antitumor immune response. Therapies that stimulate immunity have produced superior progression-free survival compared with conventional chemotherapy, reinforcing the importance of addressing the immune deficient state in the care of patients with CTCL. Recent research has better defined the pathogenesis of these immune deficits, explaining the mechanisms of disease progression and revealing potential therapeutic targets. The features of the malignant cell in mycosis fungoides and Sézary syndrome are now significantly better understood, including the T helper 2 cell phenotype, regulatory T cell cytokine production, immune checkpoint molecule expression, chemokine receptors, and interactions with the microenvironment. The updated model of CTCL immunopathogenesis provides understanding into clinical progression and therapeutic response. [ABSTRACT FROM AUTHOR]

In the past few decades, immunotherapy has emerged as an effective therapeutic option for patients with cutaneous T cell lymphoma (CTCL). CTCL is characterized by progressive impairment of multiple arms of the immune system. Immunotherapy targets these deficits to stimulate a more robust antitumor response, thereby both clearing the malignant T cells and repairing the immune dysfunction. By potentiating rather than suppressing the immune system, immunotherapy can result in longer treatment responses than alternatives such as chemotherapy. In recent years, advances in our understanding of the pathogenesis of CTCL have led to the development of several new agents with promising efficacy profiles. The second article in this continuing medical education series describes the current immunotherapeutic options for treatment of CTCL, with a focus on how they interact with the immune system and their treatment outcomes in case studies and clinical trials. We will discuss established CTCL immunotherapies, such as interferons, photopheresis, and retinoids; emerging therapies, such as interleukin-12 and Toll-like receptor agonists; and new approaches to targeting tumor antigens and checkpoint molecules, such as mogamulizumab, anti-programmed cell death protein 1, anti-CD47, and chimeric antigen receptor T cell therapy. We also describe the principles of multimodality immunotherapy and the use of total skin electron beam therapy in such regimens. [ABSTRACT FROM AUTHOR]

Background: T-cell receptor (TCR) clonality may help establish a diagnosis of mycosis fungoides (MF). Routine clonality analysis is performed by using a polymerase chain reaction TCR- gamma assay, yet with this method, 10% to 50% of T-cell lymphomas escape detection. TCR- beta gene rearrangement is an additional assay. Data about its efficacy are controversial.Objective: To evaluate the role of TCR-β assay in the diagnosis of early MF.Methods: A retrospective study of 61 skin biopsies, 20 from patients with MF, 30 from patients suspected to have early MF, and 11 from patients with chronic inflammatory skin disease.Results: Monoclonality was detected in 16 of 20 (80%) MF cases: 15 (75%) with TCR-β and 12 (60%) with TCR-γ assay. Of the 30 suspected cases of early MF, 14 showed monoclonality with TCR-β, and only 5 of 14 showed monoclonality with TCR-γ assay. None of the chronic inflammatory condition samples showed monoclonality. Therefore, TCR-β clonality assay was more sensitive than TCR-γ in early MF (83% vs 43%; P = .002).Limitations: This was a retrospective, relatively small study.Conclusion: TCR-β showed a higher sensitivity rate compared with TCR-γ in early-stage MF. The combined use of the TCR-β and TCR-γ clonality tests can significantly improve the diagnosis rate of early-stage MF. [ABSTRACT FROM AUTHOR]

Background: Mycosis fungoides (MF) is associated with increased risk of second primary hematologic malignancies, but its association with second primary solid tumors is less well characterized.Objective: This retrospective analysis seeks to assess the risk of being diagnosed with a second primary hematologic or solid malignancy in patients with MF.Design: We performed an analysis of patients diagnosed with MF from 2000 through 2015 in the United States cancer registries of SEER-18 (N = 6742).Results: Relative risks were estimated by using standardized incidence ratios (SIRs). Among 6742 patients, there were 511 (7.5%) second cancer events (SIR, 10.15; 95% confidence interval [CI], 9.29-11.07). These included 184 (36.0%) hematologic malignancies (SIR, 39.71; 95% CI, 34.05-46.05) and 327 (64.0%) solid tumor malignancies (SIR, 7.33; 95% CI, 6.56-8.17). Patients with MF were at increased risk for non-Hodgkin lymphoma; Hodgkin lymphoma; melanoma; and lung, female breast, prostate, colon, and renal cancers. Females were at higher risk than males (P < .05). All ethnic groups showed a statistically significant elevation in SIRs. Elevation of SIRs was observed across all stages of MF.Conclusions and Relevance: Patients with MF are at increased risk for diagnosis of second primary malignancies and should be carefully screened for discernable signs and symptoms of second malignancies. [ABSTRACT FROM AUTHOR]

Background: Low-dose total skin electron beam therapy (TSEBT) for mycosis fungoides is popular because of reduced toxicity with effective palliation. We condensed TSEBT, reducing visits by half and overall treatment length by one third.Objective: To determine the efficacy and safety of a novel condensed low-dose TSEBT for mycosis fungoides.Methods: We conducted a cohort study (2014-2018) with a median follow-up of 22.8 months. We delivered 12 Gy per 6 fractions with the modified Stanford technique, 3 fractions per week, with boosts to shadowed sites at risk between treatments, completing in 2 weeks. Primary outcomes included clinical response, duration of and time to response, and toxicity. Secondary outcomes included patient-reported quality of life (pain, pruritus, and Dermatology Life Quality Index) and physician-scored disease burden (body surface area involvement and Modified Skin Weighted Assessment Tool).Results: Of 25 patients, stage IB was most common at the time of TSEBT (36%). The overall response rate was 88%. Most common was a near complete response (36%), and complete response was achieved in 6 (24%) patients. The median duration of response was 17.5 months (3.5-44.2), and the median time to response was 2 months (range, 0.9-4.1). No patients had toxicity of grade 3 or greater. QOL and disease burden showed significant benefit after TSEBT (P < .001).Limitations: Cohort study with limited sample size.Conclusions: Condensed, low-dose TSEBT has favorable outcomes and toxicity with logistical convenience. [ABSTRACT FROM AUTHOR]

Genomic analysis of cancer offers the hope of identifying new treatments or aiding in the selection of existing treatments. Rare leukemias pose additional challenges in this regard as samples may be hard to acquire and when found the underlying pathway may not be attractive to drug development since so few individuals are affected. In this case, it can be useful to identify common mutational overlap among subsets of rare leukemias to increase the number of individuals that may benefit from a targeted therapy. This chapter examines the current mutational landscape of large granular lymphocyte (LGL) leukemia with a focus on STAT3 mutations, the most common mutation in LGL leukemia to date. We examined the linkage between these mutations and autoimmune symptoms and disorders, in cases of obvious and suspected LGL leukemia. We then summarized and compared mutations in a set of other rare leukemias that also have JAK/STAT signaling pathway activation brought about by genomic changes. These include T-cell acute lymphoblastic leukemia (T-ALL), T-cell prolymphocytic leukemia (T-PLL), cutaneous T-cell lymphoma (CTCL), select peripheral T-cell lymphoma (PTCL), and adult T-cell leukemia/lymphoma (ATLL). Though STAT3 activation is common in these leukemias, the way in which it is achieved, such as the activating cytokine pathway and/or the co-mutational background, is quite diverse. [ABSTRACT FROM AUTHOR]

Follicular mucinosis (FM), which is defined by mucin accumulation within follicular epithelium, may occur in mycosis fungoides (MF). FM without MF is occasionally reported in systemic hematologic malignancies and may be diagnostically challenging. To describe clinicopathologic characteristics of FM in patients with hematologic malignancies other than MF. Clinical data and histopathology features were analyzed in patients with FM and hematologic malignancies diagnosed between 1994 and 2017. A total of 18 patients with FM and systemic hematologic malignancies without cutaneous T-cell lymphoma (CTCL) were identified; 9 of them were discovered after hematopoietic stem cell transplantation. No patients with non–CTCL-associated FM (n = 46 [37 biopsy specimens]) developed CTCL during a mean follow-up of 4.3 years. Of the cases of CTCL associated with FM (n = 44 [31 biopsy specimens]), MF was the most common subtype (n = 38), although other CTCLs were identified. FM in patients with non-CTCL hematologic malignancies differed clinically from those with MF-associated FM, presenting most frequently with erythematous papules (P <.0001), without plaques (P <.0001), without alopecia (P =.001), and without histopathologically identified epidermal exocytosis (P =.013). A retrospective study in a single cancer center. FM can present in systemic hematologic malignancies, including after hematopoietic stem cell transplantation. Papular lesional morphologic and histopathologic features may help to distinguish these cases from MF. [ABSTRACT FROM AUTHOR]

OBJECTIVE: The first report concerning methotrexate (MTX) in the treatment of Mycosis fungoides (MF) was published in 1964 by Wright. The mechanism of MTX action in the treatment of primary cutaneous T-cell lymphoma (CTCL) has been not explained in detail yet (the anti-inflammatory, immunomodulating, immunosuppressive, and cytostatic actions have been under discussion). PATIENTS AND METHODS: This is a retrospective analysis of 79 MF patients in 4 dermatology clinical centers in Poland. Data are presented in terms of the duration, use of MTX, the effectiveness of treatment with MTX in terms of time required to achieve remission, the disease stage, route of administration, age at diagnosis and the dosage. Moreover, the occurrence of side effects depending on the route of administration and duration of therapy with MTX was analyzed. RESULTS: The analysis has revealed that 56 patients (70,9%) had achieved remission on the MTX. The remission began in the 1st month of therapy in 20% of patients, lasted 4 to 6 months in 50% of cases. At least 12 months' remission was confirmed in 25% of patients (2-year-long only in 10% and 3-year-long in 5% of patients). The time to remission was related to the stage of disease at diagnosis as well as to minimal and maximal dose of MTX. The total therapeutic dose of MTX was found important for the course of the disease: higher total dose had prolonged the remission. CONCLUSIONS: Despite the common use of MTX in MF patients, relatively few clinical studies have been published. The response of MF subjects to MTX seems to depend on the stage and, more importantly, the dose of MTX treatment. Methotrexate appears to be an effective treatment at every stage of MF; however, it is not devoided of side effects such as infections and elevated level of aminotransferases, which are most common. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate patterns of cannabis use in patients with cutaneous lymphoma (CL), as well as the association between cannabis use and itch specifically.Design: Cross-sectional survey created in partnership with the Cutaneous Lymphoma Foundation (CLF).Setting: The online survey was distributed electronically via email to the CLF listserv and links posted to social media over a 2-week period.Main Outcome Measures: Respondents were classified as current cannabis users, prior users, and never users. A visual analog scale (VAS) was used to assess itching severity, improvement of itch, and interest in learning about cannabis.Results: A total of 119 patient responses (61% female, mean age 59 y) were included in analysis. The majority had mycosis fungoides or Sézary syndrome (74%; 88/119) and early stage (IA-IIA) disease (56%; 48/86). Mean VAS itch score was 3.2 + 2.8 for the cohort. Over half (55%; 60/110) reported ever having used cannabis, with 22% (24/110) endorsing current cannabis use. Common methods of cannabis use were smoking (54%) and vaporizing (46%). 25% (6/24) of current users reported using cannabis specifically to treat itch; these respondents noted that cannabis resulted in moderate improvement of itching (mean 6.6/10). There was strong interest in learning more about cannabis and cancer, and most desired this information from their CL doctor/nurse.Conclusions: Cannabis use is common among patients with CL, and patients report improvement of itching as a result of using cannabis. Further studies are needed to elucidate the risks and benefits of cannabis use in this patient population. [ABSTRACT FROM AUTHOR]

Abstract: Aim: The aim of this paper was to present diagnostic methods helping in the recognition of mycosis fungoides (MF) and Sezary syndrome (SS). Background: Mycosis fungoides is the most common form of primary cutaneous T-cell lymphomas. It is characterized by a distinctive long-term course and malignant T-cell proliferation. MF diagnosis is not easy, mainly due to the atypical clinical presentation of the disease at an early stage. Materials and methods: Low specific changes, which can be observed at the histopathological examination. Initially, the skin lesions may resemble psoriasis, atopic dermatitis or chronic eczema. Patients are qualified according to the available, and generally accepted WHO-EORTC classification, based on a combination of clinical and histopathological markers. From a clinical point of view, it is also important to carry out the qualification according to the TNMB assessment, which allows to specify the stage of the disease, and is helpful in the monitoring of the course of disease and therapeutic effects. Results: In this paper we try to present currently available diagnostic methods. Conclusion: Diagnosis of MF and SS still causes many problems due to less characteristic changes in the early stage of disease and requires wide interdisciplinary knowledge. [Copyright &y& Elsevier]

Abstract: This article describes the achievements of the Cutaneous LymphomaTask Force (CLTF) over the recent decade in their goal to optimize classification and response criteria and establish new treatment options for patients suffering from cutaneous T-cell lymphomas (CTCL). Collaborative work with the International Society of Cutaneous Lymphoma (ISCL) and the United States Cutaneous Lymphoma Consortium (USCLC) has led to publication of pivotal manuscripts proposing revised staging proposals for Mycosis fungoides (MF)/Sézary syndrome (SS) and also non MF/SS primary cutaneous lymphomas as well as the recent publication of a proposal for defining endpoints in MF/SS. [Copyright &y& Elsevier]

Abstract: Mycosis fungoides is the most common type of Cutaneous T-cell Lymphoma. Data on clinical characteristics and disease progression in children with mycosis fungoides are limited. Five patients from the Middle East with childhood mycosis fungoides are reported here. Four of those patients presented with hypopigmented patches as the sole manifestation of the disease. Histopathologic features were consistent with mycosis fungoides in all patients and in four of five patients CD4+ phenotype was over-represented immunohistochemically. All patients had excellent prognosis. Only one patient with the classic erythematous patch/plaque type progressed to stage IIA. It seems the phenotype expression with either CD4+ or CD8+ has no implication on disease progression. However, the clinical morphologic type might impact disease progression with the hypopigmented type being the variant with the best prognosis. Further studies with large number of patients are required for better characterization of Middle Eastern patients with childhood MF. [Copyright &y& Elsevier]

Abstract: MicroRNAs (miRNAs) are small RNA species that regulate gene expression post-transcriptionally and are aberrantly expressed in many malignancies including lymphoma. However, the role of miRNAs in the pathogenesis of T-cell lymphoid malignancies is poorly understood. Previously we examined the miRNA profile of Sézary syndrome (Sz), a leukemia of skin-homing memory T cells. In this study we determined the complete miRNome of mycosis fungoides (MF), the most common type of cutaneous T cell lymphoma. The miRNA profile of skin biopsies from 19 patients with tumor stage MF and 12 patients with benign inflammatory dermatoses (eczema and lichen planus) were compared by microarray analysis. We identified 49 miRNAs that are differentially expressed in tumor stage MF compared to benign inflammatory dermatoses using ANOVA analysis (P < 0.05, Benjamini–Hochberg corrected). The majority of the differentially expressed miRNAs (30/49) were up-regulated in tumor stage MF. The most significant differentially expressed were miR-155 and miR-92a (both up-regulated in tumor stage MF), while miR-93 showed the highest up-regulation in tumor stage MF with a fold difference of 5.8. Differential expression of a selection of these miRNAs was validated by miRNA-Q-PCR on additional test groups (tumors and controls). None of the miRNAs up-regulated in tumor stage MF was previously shown to be up-regulated in Sz, and only 2 of the 19 miRNAs down-regulated in tumor stage MF were also down-regulated in Sz. Taken together this report is the first describing the miRNA signature of tumor stage MF. [Copyright &y& Elsevier]

Background: The role of dendritic cells (DCs) in disease progression of primary cutaneous T-cell lymphoma (CTCL) is not well understood. With their unique ability to induce primary immune responses as well as immunotolerance, DCs play a critical role in mediation of anti-tumor immune responses. Tumor-infiltrating DCs have been determined to represent important prognostic factors in a variety of human tumors. Objective: To date, only circulating DC populations have been investigated in CTCL. Therefore we analyzed the expression and tissue distribution of different DC subset markers in lesional and nonlesional skin of patients with CTCL. Methods: Twelve patients with mycosis fungoides or Sézary syndrome were included in the study. Tissue samples were obtained from lesional and nonlesional skin as a control. Immunohistochemistry was performed with different DC subset and regulatory T-cell markers and assessed using a digital image analysis system. Tissue distribution of DCs in relation to the tumor was analyzed by double immunofluorescence. Results: We found a significant infiltration of CTCL lesions by immature CD209/DC-SIGN+ DCs with close contact to tumor cells. Matured and activated DCs were only rarely detected in lesions of CTCL. Limitations: The sample size was small. Conclusions: The preponderance of immature CD209/DC-DIGN+ DCs in contact with regulatory T cells in lesions of CTCL points to an important role of this subset in the host''s immune reaction to the malignant T cells. Since these immature DCs are known to induce immunotolerance, they might play a role in the mediation of immune escape of the proliferating clone. [Copyright &y& Elsevier]

Summary: Primary cutaneous T-cell lymphomas are a heterogenous group of non-Hodgkin lymphomas. The characteristic clinicopathologic and immunophenotypic features and prognoses of the various cutaneous lymphomas have been recently described by the World Health Organization and European Organization for Research and Treatment of Cancer. Cutaneous T-cell lymphoma variants include mycosis fungoides and Sezary syndrome, which are generally associated, respectively, with indolent and aggressive clinical courses and are the subject of this review. Currently utilized treatments for cutaneous T-cell lymphoma include skin-directed therapies (topical agents such as corticosteroids, mechlorethamine, carmustine, and retinoids, phototherapy, superficial radiotherapy, and total skin electron beam therapy), systemic therapies (photophoresis, retinoids, denileukin diftitox, interferons, and chemotherapy), and stem cell transplantation (autologous and allogeneic). This review will describe recent advances in our understanding of the biology (immunologic, cytogenetic, and genetic) of cutaneous T-cell lymphomas and discuss the efficacy and tolerability of the current therapeutic options for cutaneous T-cell lymphomas. Disease progression in over 20% of patients with early stages of disease and the current lack of a definitive treatment which produces durable responses in advanced stages of disease indicates a critical unmet need in CTCL. New insights into the molecular and immunologic changes associated with cutaneous T-cell lymphomas should ultimately lead to the identification of novel therapeutic targets and the development of improved therapeutic options for patients with these malignancies. [Copyright &y& Elsevier]

Objectives: To characterize patterns of integrative medicine (IM) use and health-related quality of life (HRQoL) in patients with cutaneous T-cell lymphoma (CTCL).Design: Cross-sectional, online survey, created in conjunction with the Cutaneous Lymphoma Foundation (CLF).Setting: A link to the online survey was posted on the CLF Facebook page and emailed to the CLF listserv; 372 survey responses were received.Main Outcome Measures: The study outcomes were IM use, cancer symptoms, and HRQoL measured via the Skindex-16 and Functional Assessment of Cancer Therapy-General (FACT-G).Results: A total of 292 patient responses (66 % female, median age 59y) were included in analysis. 87 % had mycosis fungoides and 12 % had Sézary syndrome. A majority (59 %) of patients reported using IM for their CTCL, with 48 % using IM to treat their disease and 47 % using IM to manage their symptoms. The most commonly used IM were vitamins/minerals (32 %), prayer/meditation (26 %), diet (24 %), and exercise/yoga (22 %). Higher itch scores were reported by patients using IM compared to non-users (31 (IQR 10-62) and 18 (IQR 3-46) respectively; p = 0.002). HRQoL was worse among patients who reported IM use; median Skindex-16 scores were 54 (IQR 28-72) among IM users compared to 33 (IQR 19-57) for non-IM users (p < 0.001).Conclusions: IM use is common among patients with CTCL, particularly those with worse itching and worse HRQoL. IM interventions require further study given use by CTCL patients to treat disease and ameliorate symptoms. [ABSTRACT FROM AUTHOR]

Summary: Among patients with cutaneous T-cell lymphoma (CTCL), sepsis and pulmonary infections are the first cause of death. We report on a patient with CTCL who, after more than 10 years of aggressive antineoplastic treatments, showed extensive pulmonary infiltrations on staging CT scan. Repeated CT scans were inconclusive for an infectious process, and the patient was still asymptomatic. The diagnosis of mycobacteriosis was made on the microbiologic exam of bronchoalveolar lavage. Specific treatment was started with contemporary dosage reduction of chemotherapy. After six months of antibiotic treatment the pulmonary lesions improved, whereas CTCL progressed. Therefore, a new antineoplastic regimen was started obtaining control of CTCL, without aggravation of the pulmonary lesions. We highlight the diagnostic and therapeutic pitfalls encountered when pulmonary mycobacteriosis complicates the course and treatment of CTCL. [Copyright &y& Elsevier]

Background: Follicular mucinosis (FM), which is defined by mucin accumulation within follicular epithelium, may occur in mycosis fungoides (MF). FM without MF is occasionally reported in systemic hematologic malignancies and may be diagnostically challenging.Objective: To describe clinicopathologic characteristics of FM in patients with hematologic malignancies other than MF.Methods: Clinical data and histopathology features were analyzed in patients with FM and hematologic malignancies diagnosed between 1994 and 2017.Results: A total of 18 patients with FM and systemic hematologic malignancies without cutaneous T-cell lymphoma (CTCL) were identified; 9 of them were discovered after hematopoietic stem cell transplantation. No patients with non-CTCL-associated FM (n = 46 [37 biopsy specimens]) developed CTCL during a mean follow-up of 4.3 years. Of the cases of CTCL associated with FM (n = 44 [31 biopsy specimens]), MF was the most common subtype (n = 38), although other CTCLs were identified. FM in patients with non-CTCL hematologic malignancies differed clinically from those with MF-associated FM, presenting most frequently with erythematous papules (P < .0001), without plaques (P <.0001), without alopecia (P = .001), and without histopathologically identified epidermal exocytosis (P = .013).Limitations: A retrospective study in a single cancer center.Conclusions: FM can present in systemic hematologic malignancies, including after hematopoietic stem cell transplantation. Papular lesional morphologic and histopathologic features may help to distinguish these cases from MF. [ABSTRACT FROM AUTHOR]